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The lymphoreticular system and bone marrow
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Thymoma is a primary epithelial tumour of the thymus that behaves like a low-grade carcinoma, invading surrounding structures but seldom metastasizing (<10% of cases). The lesion consists of epithelial cells, often interspersed with so many thymic lymphocytes that it can be misdiagnosed as a lymphoma. Although often found incidentally, thymoma may be associated with myasthenia gravis, pure red cell aplasia, hypogammaglobulinaemia, and autoimmune diseases such as polymyositis. Several forms of lymphoma may affect the thymus, most notably classical Hodgkin lymphoma and mediastinal large B-cell lymphoma, both typically in young females. T-lymphoblastic leukaemia, which usually occurs in childhood, may present with a large anterior mediastinal mass. Germ cell tumours, similar to those found in the gonads, can arise at this site.
Post-Transplant Lymphoproliferative Disorders (PTLD)
Published in Dongyou Liu, Tumors and Cancers, 2017
Grouped together with mature B-cell neoplasms, mature T and NK neoplasms, Hodgkin lymphoma, and histiocytic and dendritic cell neoplasms under the mature lymphoid, histiocytic, and dendritic neoplasms category, post-transplant lymphoproliferative disorders (PTLD) consist of six types: (i) plasmacytic hyperplasia PTLD (PH-PTLD), (ii) infectious mononucleosis PTLD (IM-PTLD), (iii) florid follicular hyperplasia PTLD (FFH-PTLD), (iv) polymorphic PTLD (P-PTLD), (v) monomorphic PTLD (B- and T-/NK-cell types) (M-PTLD), and (vi) classical Hodgkin lymphoma PTLD (CHL-PTLD) (Table 26.1) [1].
Paediatric oncology
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2014
Stephen Lowis, Rachel Cox, John Moppett, Antony Ng
Histologically, paediatric HL is classified according to the Rye classification, now incorporated into the WHO classification.41 It is sub-classified into two disease entities, classical Hodgkin lymphoma (CHL) and nodular lymphocyte predominant Hodgkin lymphoma (NLPHL). CHL has several subtypes: lymphocyte rich, nodular sclerosing, mixed cellularity and lymphocyte depleted. Mixed cellularity type CHL (which has a better prognosis) is more common in children than adults, but lymphocyte-depleted CHL is uncommon. NLPHL has a different cell of origin, clinical course and response to treatment than CHL, so it is considered separately (see the section ‘Nodular Lymphocyte Predominant Hodgkin Lymphoma’).
Rapid T-cell lymphoma progression associated with immune checkpoint inhibitors
Published in Expert Review of Hematology, 2023
Immunotherapy is the fourth pillar of cancer treatment, and immune checkpoint inhibitors (ICIs) are one of its branches. Considering the striking clinical features of melanoma or lung cancer, ICIs are currently prescribed to patients with various types of solid malignancies in clinical practice [1]. In cases of hematological malignancies, classical Hodgkin lymphoma (CHL) is the representative indication. Phase 2 trials of anti-programmed death-1 (PD1) antibody agents pembrolizumab or nivolumab for patients with relapsed/refractory CHL have exhibited overall response rates (ORRs) of 69% and 66%, respectively [2,3]. These results seem outstanding in that many enrolled patients experienced treatment failure after autologous hematopoietic stem-cell transplantation (ASCT). A phase 2 trial of nivolumab plus brentuximab vedotin for relapsed/refractory CHL patients in a post-ASCT consolidation setting has recently shown a progression-free survival rate of 94% at 18 months [4]. In addition, anti-PD1 antibodies are a promising treatment for relapsed/refractory primary mediastinal B-cell lymphoma (PMBCL). The rationale involves elevated programmed death-1 ligand (PD-L1) expression in PMBCL. Two trials of pembrolizumab showed ORRs of 48% and 45% [5].
Core needle biopsy is an inferior tool for diagnosing cervical lymphoma compared to lymph node excision
Published in Acta Oncologica, 2021
Rasmus Krarup Sigaard, Kasper Wennervaldt, Lars Munksgaard, Lise Mette Rahbek Gjerdrum, Preben Homøe
The final histopathological diagnoses encompassed 44 cases of lymphoma, six nonmalignant hyperplasias, four sarcoidosis, and two non-lymphoma malignancies of which one was a metastasis from a seminoma and one was a myeloid sarcoma (Table 1). In 41 cases (73%), the CNB and SEB-res diagnoses were in agreement (Table 2). There was full agreement in cases with reactive histology, sarcoidosis, T-cell lymphomas, and non-lymphoma malignancies. In cases with classical Hodgkin lymphoma, only two of six cases were conclusive or suggestive of the final diagnosis. In the B-cell lymphoma group, 25 of the 36 cases resulted in the same diagnoses (69%), and 11 cases diverged (Table 1). In four cases, the CNB was inconclusive or gave a false diagnosis. In one case, the CNB revealed changes suspicious for B-cell lymphoma, but were not conclusive.
Identifying and treating candidates for checkpoint inhibitor therapies in multiple myeloma and lymphoma
Published in Expert Review of Hematology, 2020
Katarina Hradska, Michal Kascak, Roman Hajek, Tomas Jelinek
Classical Hodgkin lymphoma is characterized by the presence of malignant RS cells and the extensive inflammatory microenvironment of immune cells (represented mostly by T cells and macrophages) [65]. RS cells escape immune detection and elimination by hijacking PD-1/PD-L1/2 pathway and with the downregulation of MHC. Upregulation of PD-L1/PD-L2 and JAK2 is driven by almost uniform 9p24.1 genetic alteration (copy gain, amplification) in RS cells and frequent EBV infection in cHL patients [65,68]. Overexpression of PD-1 ligands is more common in the advanced stages of cHL and is associated with inferior PFS [68]. Extensive expression of PD-1 ligands on RS cells, on surrounding macrophages and the simultaneous presence of PD-1 positive tumor-infiltrating lymphocytes (TIL) make cHL an attractive target for checkpoint inhibitor therapy [67].